Wongwaisayawan S, Pornkul R, Teeraratkul S, Pakakasama S, Treepongkaruna S, Nithiyanant P
Department of Pathology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
J Med Assoc Thai. 2000 Mar;83 Suppl 1:S17-22.
The authors report a case of beta-thalassemia/hemoglobin E disease with extramedullary haematopoietic tumor which developed at the small intestine and caused intussusception. A 7 year-old boy with homozygous beta-thalassemia/hemoglobin E presented with recurrent abdominal pain. The abdominal ultrasonography showed ileo-ileal intussusception with a solid mass as the leading point. Resection of the ileal segment was performed. Pathological examination revealed an extramedullary haematopoietic tumor forming an intraluminal polypoid mass, being the leading point of the intussusception. Extramedullary haematopoiesis in the intestinal tract is rare. To our knowledge, this is the first case of extramedullary haematopoietic tumor that produced intussusception of the small intestine in a beta-thalassemia/hemoglobin E patient.
作者报告了一例β地中海贫血/血红蛋白E病合并髓外造血肿瘤的病例,该肿瘤发生于小肠并导致肠套叠。一名患有纯合子β地中海贫血/血红蛋白E的7岁男孩出现反复腹痛。腹部超声显示回肠-回肠型肠套叠,以实性肿块为套入点。进行了回肠段切除术。病理检查显示髓外造血肿瘤形成腔内息肉样肿块,为肠套叠的套入点。肠道髓外造血较为罕见。据我们所知,这是首例β地中海贫血/血红蛋白E患者发生的导致小肠肠套叠的髓外造血肿瘤病例。